3.2 Qualitative Platelet Disorders (Vascular disorder) Flashcards
Hereditary vascular disorder includes:
- Allergic purpura
- Paraproteinemia and Amyloidosis
- Senile purpua
- Drug-induced vasculitis
- Vitamine C deficiency
T or F
F
Acquired
Also known as Hereditary Hemorrhagic telangectasia
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
a
Also known as Hemangioma Thrombocytopenia syndrome
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
b
Presense of giant cavernous vasuclar tumor, thrombocytopenia and bleeding diathesis
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
b
Characterized by thin-walled blood vessels with a discontinuous endothelium
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
a
Also known as hypermobility syndrome
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
c
Disorder is due to defects in collagen production, structure or cross linking with resulting inadequacy of the connective tissues
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
c
A progressive disorder that is characterized by the accumulation of deposits of calcium and other minerals in elastiv fiber
a. Rendu-Osler-Weber syndrome
b. Kasabac-Merritt Syndrome
c. Ehlers-Danlos Syndrome
d. Pseudoxanthoma elasticum
d
Caused by defect in the gene that encodes the structure of fibrillin and elastic fivers, a major component of connective tissue
a. Marfan syndrome
b. Osteogenesis imperfecta
a
Hereditary connective tissue disease often due to mutations in genes coding for type 1 collagen
a. Marfan syndrome
b. Osteogenesis imperfecta
b
Nonthrombocytopenic purpuras characterized by allergic manifestations
a. Allergic purpura
b. Paraproteinemia and Amylodosis
c. Senile purpura
d. Drug-induced vasculitis
e. Vitamin C deficiency (Scurvy)
f. Henoch-Schonlein purpura
a
An Acute IgA-Mediated disorder with widespread generalized vasculitis involving the skin, joints, kidneys, gastrointestinal tract and, less commonly, the lungs
a. Allergic purpura
b. Paraproteinemia and Amylodosis
c. Senile purpura
d. Drug-induced vasculitis
e. Vitamin C deficiency (Scurvy)
f. Henoch-Schonlein purpura
f
in Allergic purpura, the Vasculitis is mediated by immune complexes containing Ig_ antibodies
A
High concentrations of this can cause severe hemorrhagic manifestations as a result of a combination of hyper viscosity and platelet dysfunction
a. Allergic purpura
b. Paraproteinemia and Amylodosis
c. Senile purpura
d. Drug-induced vasculitis
e. Vitamin C deficiency (Scurvy)
f. Henoch-Schonlein purpura
Paraprotein
It is the deposition of abnormal quantities of amyloid protein in tissues and may result to thrombosis and hemorrhage
Amyloidosis
More commonly seen in elderly men than in women
Due to lack of collagen support for blood vessels and loss of subcutaneous fat and elastic fibers
a. Allergic purpura
b. Paraproteinemia and Amylodosis
c. Senile purpura
d. Drug-induced vasculitis
e. Vitamin C deficiency (Scurvy)
f. Henoch-Schonlein purpura
c
Mechanisms include development of Ab to vessel wall components, development of immune complexes and changes in vessel wall permeability
a. Allergic purpura
b. Paraproteinemia and Amylodosis
c. Senile purpura
d. Drug-induced vasculitis
e. Vitamin C deficiency (Scurvy)
f. Henoch-Schonlein purpura
d
Deficiency in ascorbic acid’s role in collagen synthesis
Scurvy
